Asadi Gharabaghi et al.

Journal of Medical Case Reports 2011, 5:235

http://www.jmedicalcasereports.com/content/5/1/235

JOURNAL OF MEDICAL

CASE REPORTS

CASE REPORT

Open Access

Systemic lupus erythematosus presenting

with eosinophilic enteritis: a case report
Mehrnaz Asadi Gharabaghi1*, Pejman Abdollahi 2, Mohammad Kalany3 and Masoud Sotoudeh4

Abstract
Introduction: Systemic lupus erythematosus (SLE) is a multisystem disorder that may present with various
symptoms. It may involve the gastrointestinal tract in a variety of ways; some of the most well-known ones are
transaminitis, lupus mesenteric vasculitis, lupus enteritis and mesenteric vascular leakage. We describe a case of a
patient with SLE who presented with a five-month history of diarrhea caused by eosinophilic enteritis. To the best
of our knowledge, there are few cases reported in the literature of patients with SLE who initially present with
chronic diarrhea due to eosinophilic enteritis.
Case presentation: A 38-year-old Persian Iranian woman was admitted with a five-month history of diarrhea and
abdominal pain. A physical examination showed nothing abnormal. Initially, she had only lymphopenia and mild
eosinophilia. No autoimmune or infectious etiology was detected to justify these abnormalities. A thorough
evaluation was not helpful in finding the etiology, until she developed a scalp lesion similar to discoid lupus
erythematosus. Computed tomography showed small bowel wall thickening. Briefly, she manifested full-blown SLE,
and it was revealed that the diarrhea was caused by eosinophilic enteritis.
Conclusion: Considering SLE in a patient who presents with chronic diarrhea and lymphopenia may be helpful in
earlier diagnosis and therapy. This is an original case report of interest to physicians who practice internal
medicine, family medicine and gastroenterology.

Introduction
Systemic lupus erythematosus (SLE) is the prototypical
autoimmune disease caused by auto-antibodies that
recognize self-antigens as foreigners. These pathogenic
antibodies are produced against the components of the
cell nucleus. The abnormal immune response results in
target tissue injury, and the ensuing inflammatory reaction deranges various organ functions. Nearly every
organ system may be involved in the disease course. It
may present initially with non-specific symptoms or atypical manifestations, so the diagnosis may present a considerable challenge at the early stages [1-3]. In this way,
it is not surprising that a patient who is initially diagnosed with acute inflammatory demyelinating polyneuropathy will later be found to have SLE [4]. SLE may
involve the gastrointestinal tract in a variety of ways.
Some of the most common manifestations are
* Correspondence: asadi_m@tums.ac.ir
1
Department of General Internal Medicine, Imam Khomeini Hospital
Complex, Tehran University of Medical Sciences, Tehran, Iran
Full list of author information is available at the end of the article

dyspepsia, hepatitis, mesenteric vasculitis, enteritis and

mesenteric vascular leakage [5-8]. However, to our
knowledge, there are few cases of the disease in which
the patient initially presented with chronic diarrhea due
to eosinophilic enteritis. Herein we report the case of
38-year-old woman who presented with a five-month
history of diarrhea and abdominal pain and was also
diagnosed with SLE.

Case presentation
A 38-year-old Persian Iranian woman was admitted to
our hospital to be evaluated for abdominal pain and
diarrhea. Overall, she had been well until five months
before her presentation. Thereafter she noticed a vague,
constant pain over her abdomen. At the time of her
admission to another hospital, the work-up culminated
in a laparatomy. Her right ovary contained a hemorrhagic cyst that was resected. It was a benign ovarian cyst.
She was then discharged. However, her abdominal pain
persisted. A few days later she noticed frequent bowel
movements with large amounts of watery stool each

2011 Mehrnaz et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative
Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and
reproduction in any medium, provided the original work is properly cited.

Asadi Gharabaghi et al. Journal of Medical Case Reports 2011, 5:235

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time. Occasionally, a sense of flushing also bothered her.

The diarrhea persisted for more than two weeks without
any change in spite of fasting or changes in food intake.
She also experienced anorexia and weight loss. She underwent a through work-up at another hospital. Computed
tomography (CT) of her abdomen was performed, which
revealed thickening of the ascending bowel wall (Figure 1).
Thereafter she was referred to our hospital to undergo
further follow-up to rule out carcinoid syndrome.
She was a young woman concerned about her disease.
Except for diarrhea and abdominal pain, she did not complain about fever, fecal incontinence or extra-intestinal
manifestations. Her physical examination revealed pale
conjunctivae and an enlarged spleen suggested by a dull
percussion note on Traubes semilunar space. There were
also several groups of slightly enlarged cervical and inguinal lymph nodes that were soft, mobile and non-tender.
She had no family history of inflammatory bowel disease or celiac disease. On the basis of the results of
stool analyses and microbiologic studies, her condition
was diagnosed as non-inflammatory diarrhea.
Since she had a CA-125 level to a high of 138 U/l
(normal, up to 37 U/l), histological reexamination of

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tissues removed during her prior surgery was performed.

The result was the same as the previous finding. The
elevated level of CA-125 was attributed to a benign
ovarian cystic disorder. Routine blood studies identified
normochromic normocytic anemia, lymphopenia (hemoglobin level, 11.0 g/dl; lymphocyte count, 960/l) and
hypoalbuminemia (2.8 g/dl). There was no elevation in
her erythrocyte sedimentation rate (25 mm/hour) or Creactive protein level. Her blood chemistry showed no
sign of electrolyte, hepatic or other metabolic disturbances. Neither nutritional deficiency nor blood loss was
identified as the contributing factor to her anemia. Her
serum ferritin level was 379 ng/ml (reference interval,
18 n g/ml to 341 ng/ml). An examination of a blood
smear verified the presence of lymphocytopenia. Ultrasonography of the abdomen revealed an ovarian cyst in
the left ovary measuring 3 cm 5 cm in addition to a
slightly enlarged spleen; otherwise, the findings were
normal.
The most frequent cause of non-inflammatory chronic
diarrhea among Iranians is celiac disease (CD) [9]. However, serologic biomarkers and endoscopic and histologic surveys of the duodenum were both inconsistent

Figure 1 Section of abdominal scan with oral contrast obtained at the time of admission showing increased thickness of the
ascending colon wall.

Asadi Gharabaghi et al. Journal of Medical Case Reports 2011, 5:235

http://www.jmedicalcasereports.com/content/5/1/235

with CD. Biopsy specimens of the normal-appearing distal colon were taken. Her histological diagnosis
inveighed against the presence of microscopic colitis.
Her thyroid function tests were normal, too. The presence of diarrhea, abdominal pain and occasional flushing mandated screening for peptide hormones.
Neuroendocrine tumors such as carcinoids might be
considered. However, the results of the hormone assay
disproved the existence of such tumors.
On the basis of our patients medical symptoms, the
differential diagnosis included SLE, intestinal lymphoma,
acquired immunodeficiency syndrome (AIDS) and postmucosal lymphatic obstruction. CT of her abdomen was
performed with both oral and IV contrast agents to rule
out structural or occult inflammatory intestinal
disorders.
A bone marrow biopsy was evaluated but yielded no
evidence of lymphoma. HIV antibody and SLE serologic
biomarkers were checked. The results of the studies
were pending at the time that she was discharged with
instructions to return the following week.
After one week, she returned to our hospital complaining of new-onset painful lesions on her scalp. Her
diarrhea and vague generalized abdominal pain persisted. She was admitted to our hospital for the second
time.
There were several discrete erythematous plaques over
her scalp containing circumscribed areas of alopecia.
The lesions were similar to those seen in patients with
discoid lupus erythematosus (DLE) or lichen planus. A
skin biopsy confirmed the histologic diagnosis of DLE.
The results of HIV and fluorescence anti-nuclear antibody tests were negative, but anti-SSA/Ro antibody was
detected in the blood in low titer. Her anti-doublestranded DNA (anti-dsDNA) antibody level was strongly
positive at 5 IU/ml (> 1.1 was considered positive).
Peripheral eosinophilia was also present. Her eosinophil count was 780/l, and her total white blood cell
count was 4,610/l. There was no evidence of parasitic
infection on the basis of repeated examination of stool
samples and serology tests to detect strongyloidosis, toxocariasis, trichinellosis, liver flukes and so on. She
reported no history of allergic disorders or intake of
over-the-counter drugs.
CT of her abdomen demonstrated only bowel wall
thickening; otherwise, the findings were normal. The
results were consistent with an inflammatory bowel disorder such as Crohns disease. The radiologist believed
that intestinal lymphoma would be an alternative diagnosis. However, the clinical diagnosis rendered was
lupus enteritis. Because of the discrepancies between the
clinical and radiological diagnoses, she underwent total
colonoscopy for the second time. Multiple biopsy specimens were also obtained from the ileum. The histologic

Page 3 of 5

findings were consistent with eosinophilic enteritis (Figures 2 and 3). The other possibilities, such as lymphoma, intestinal lymphangiectasia, vasculitis and
inflammatory bowel disease, were excluded.
During her hospital stay, she developed proteinuria to
a high of 4,800 mg/day. She was diagnosed with SLE
because her symptoms met the criteria for lymphocytopenia, proteinuria, DLE and positive anti-dsDNA antibodies. A kidney biopsy was performed. The results of a
histopathological study showed class V lupus nephritis.
Considering the involvement of two significant organs
(renal and gastrointestinal), she was treated by bolus
therapy with three daily pulses of methylprednisolone at
a dose of 1,000 mg/day followed by 0.5 mg/kg/day prednisolone. A few days later she no longer experienced
diarrhea or abdominal pain. In this way, we reached the
final diagnosis of eosinophilic enteritis in the context of
SLE that caused the patient to develop anorexia, abdominal pain and diarrhea.

Discussion
Diarrhea lasting more than four weeks mandates that
the physician excludes the presence of serious underlying pathologies. The patients medical history, physical
examination and routine blood studies often help in
establishing the mechanism of diarrhea. Hematologic
clues such as lymphocytopenia narrow the extent of the
differential diagnosis. Congenital intestinal lymphangiectasia or acquired lymphatic obstruction caused by a
tumor may be responsible for the co-existence of
chronic diarrhea and lymphocytopenia [10]. Other possible diagnoses include diarrhea caused by opportunistic
infection in patients with AIDS [11].
Lymphoma may also involve the gastrointestinal tract
in different ways. Both Hodgkins and non-Hodgkins
lymphoma may invade the gastrointestinal tract. Two of
the most common forms are mantle cell lymphoma and
mucosa-associated lymphoid tissue lymphoma. Primary
intestinal lymphomas may present with abdominal pain,
symptoms due to intestinal obstruction, bleeding and
diarrhea. Histologic examination of tissue obtained from
the involved area often results in a proper diagnosis [12].
Eosinophilia and diarrhea may occur because of parasitic infections, neoplasia, collagen vascular disorders
and eosinophilic gastroenteritis. Rheumatoid arthritis,
allergic angiitis and peri-arteritis nodosa also may cause
eosinophilia and gastrointestinal involvement [13].
Immune lymphocytopenia is also a characteristic feature of SLE that may be accompanied by diarrhea in a
variety of ways. Lupus enteritis and eosinophilic enteritis
may cause diarrhea in patients with SLE. Sometimes the
diarrhea develops as a consequence of treatment complications, such as opportunistic infections occurring
after cytotoxic therapy [5,7,8].

Asadi Gharabaghi et al. Journal of Medical Case Reports 2011, 5:235

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Figure 2 Photomicrograph of colonic mucosa showing aggregation of eosinophils in deeper areas of the lamina propria between the
tip of the mucosal glands and muscularis mucosa (hematoxylin and eosin stain; original magnification, 320).

Figure 3 Photomicrograph of colonic mucosa showing aggregation of eosinophils and exocytosis in the mucosal glandular epithelium
(hematoxylin and eosin stain; original magnification, 320).

Asadi Gharabaghi et al. Journal of Medical Case Reports 2011, 5:235

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Eosinophilic enteritis is an infiltrative disorder that is

characterized by patchy or diffuse eosinophilic infiltration of the intestinal wall. It rarely involves the entire
intestinal tract. It usually presents as a distinct syndrome such as eosinophilic esophagitis, gastritis and
enteritis. Dysphagia, dyspepsia and diarrhea have frequently been reported as the initial symptoms. Less frequently the disease may present with a focal mass,
protein-losing enteropathy, ascites and even gastric outlet obstruction. The disease may develop in the context
of another pre-existing disease such as CD or overlap
syndrome. The disease responds well to corticosteroids;
yet, there are cases that require no treatment because of
the mild, non-interfering nature of the disease [14,15].

Conclusion
To date, according to our knowledge, there have been
few case reports of SLE presenting with diarrhea due to
eosinophilic enteritis. Considering the disease in the differential diagnosis of patients presenting with diarrhea,
eosinophilia and lymphopenia may be helpful for practicing physicians who are frequently confronted with
chronic diarrhea cases. Therefore, this original case
report may be of interest to internists, family physicians
and gastroenterologists. The issue that should be taken
into account is the following: SLE is similar to many
autoimmune rheumatic diseases and may present atypically, lack a pathognomonic feature and run a dynamic
course.
Consent
Written informed consent was obtained from the patient
for publication of this case report and any accompanying images. A copy of the written consent is available
for review by the Editor-in-Chief of this journal.
Abbreviations
AIDS: acquired immunodeficiency syndrome; CD: celiac disease; CT:
computed tomography; DLE: discoid lupus erythematosus; SLE: systemic
lupus erythematosus.
Author details
1
Department of General Internal Medicine, Imam Khomeini Hospital
Complex, Tehran University of Medical Sciences, Tehran, Iran. 2Imam
Khomeini Hospital Complex, School of Medicine, Tehran University of
Medical Sciences, Tehran, Iran. 3Department of Gastroenterology, Imam
Khomeini Hospital Complex, School of Medicine, Tehran University of
Medical Sciences, Tehran, Iran. 4Department of Pathology, School of
Medicine, Tehran University of Medical Sciences, Tehran, Iran.

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Competing interests
The authors declare that they have no competing interests.
Received: 8 December 2009 Accepted: 25 June 2011
Published: 25 June 2011
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doi:10.1186/1752-1947-5-235
Cite this article as: Asadi Gharabaghi et al.: Systemic lupus
erythematosus presenting with eosinophilic enteritis: a case report.
Journal of Medical Case Reports 2011 5:235.

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Authors contributions
MA was the main physician who cared for the patient and prepared the
main draft of the manuscript. PA was the medical student who prepared the
patients medical history and physical examination. MS was the pathologist
who reviewed the histological evaluation of the samples. KM was the
gastroenterologist who performed the endoscopic procedures. All authors
approved the final manuscript.

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